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. 1981 Sep;56(9):708–714. doi: 10.1136/adc.56.9.708

Vitamin status in treated patients with cystic fibrosis.

P J Congden, G Bruce, M M Rothburn, P C Clarke, J M Littlewood, J Kelleher, M S Losowsky
PMCID: PMC1627301  PMID: 7294874

Abstract

The water-soluble (B1, B2, B6, C, folic acid) and fat-soluble vitamin (A, carotene, E, and D) status of 36 patients with cystic fibrosis was assessed and compared with a control group of 21 age-matched normal children. Twenty-seven of the patients were receiving vitamin supplements (except folic acid and vitamin E) at the time of investigation. Vitamin B1, B2, and B6 status was adequate in all patients, and there was little evidence of folic acid deficiency. Vitamin C stores might not have been adequate in some of these patients, despite daily supplements with 50 mg of the vitamin. Steatorrhoea, often severe, was present in most of them. Serum carotene and vitamin E concentrations were low in over 90% of patients and were related to the severity of steatorrhoea. Vitamin A was low in over 40% of the patients despite daily vitamin supplements of 4000 IU and correlated with the serum retinol-binding protein level. Serum 25-OH cholecalciferol was low in some patients whether or not they were receiving a daily supplement of 400 IU vitamin D. In a short-term supplementation trial with water-miscible preparations of vitamin A and E in 14 patients, the serum levels of both vitamins responded well to 2 weeks of treatment with 50 mg vitamin E and 4000 IU vitamin A. Except for serum vitamin A, which was lowest in patients with the poorest clinical grading, the other vitamins were not influenced by the clinical grade of the patients.

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Selected References

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  1. Bauernfeind J. C., Newmark H., Brin M. Vitamins A and E nutrition via intramuscular or oral route. Am J Clin Nutr. 1974 Mar;27(3):234–253. doi: 10.1093/ajcn/27.3.234. [DOI] [PubMed] [Google Scholar]
  2. Chase H. P., Long M. A., Lavin M. H. Cystic fibrosis and malnutrition. J Pediatr. 1979 Sep;95(3):337–347. doi: 10.1016/s0022-3476(79)80504-1. [DOI] [PubMed] [Google Scholar]
  3. DENSON K. W., BOWERS E. F. The determination of ascorbic acid in white blood cells. A comparison of W.B.C. ascorbic acid and phenolic acid excretion in elderly patients. Clin Sci. 1961 Oct;21:157–162. [PubMed] [Google Scholar]
  4. DOERSHUK C. F., MATTHEWS L. W., TUCKER A. S., NUDLEMAN H., EDDY G., WISE M., SPECTOR S. A 5 YEAR CLINICAL EVALUATION OF A THERAPEUTIC PROGRAM FOR PATIENTS WITH CYSTIC FIBROSIS. J Pediatr. 1964 Nov;65:677–693. doi: 10.1016/s0022-3476(64)80152-9. [DOI] [PubMed] [Google Scholar]
  5. Davies T., Kelleher J., Losowsky M. S. Interrelation of serum lipoprotein and tocopherol levels. Clin Chim Acta. 1969 Jun;24(3):431–436. doi: 10.1016/0009-8981(69)90116-8. [DOI] [PubMed] [Google Scholar]
  6. Di Sant'Agnese P. A., Talamo R. D. Pathogenesis and physiopathology of cystic fibrosis of the pancreas. Fibrocystic disease of the pancreas (Mucoviscidosis). N Engl J Med. 1967 Dec 28;277(26):1399+–1399+. doi: 10.1056/NEJM196712282772605. [DOI] [PubMed] [Google Scholar]
  7. Fabianek J., DeFilippi J., Richards T., Herp A. Micromethod for tocopherol determination in blood serum. Clin Chem. 1968 May;14(5):456–462. [PubMed] [Google Scholar]
  8. Farrell P. M., Bieri J. G., Fratantoni J. F., Wood R. E., di Sant'Agnese P. A. The occurrence and effects of human vitamin E deficiency. A study in patients with cystic fibrosis. J Clin Invest. 1977 Jul;60(1):233–241. doi: 10.1172/JCI108760. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Goodchild M. C., Sagaró E., Brown G. A., Cruchley P. M., Jukes H. R., Anderson C. M. Comparative trial of pancrex V forte and nutrizym in treatment of malabsorption in cystic fibrosis. Br Med J. 1974 Sep 21;3(5933):712–714. doi: 10.1136/bmj.3.5933.712. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Green J., Bunyan J. Vitamin E and the biological antioxidant theory. Nutr Abstr Rev. 1969 Apr;39(2):321–345. [PubMed] [Google Scholar]
  11. Hahn T. J., Squires A. E., Halstead L. R., Strominger D. B. Reduced serum 25-hydroxyvitamin D concentration and disordered mineral metabolism in patients with cystic fibrosis. J Pediatr. 1979 Jan;94(1):38–42. doi: 10.1016/s0022-3476(79)80346-7. [DOI] [PubMed] [Google Scholar]
  12. Hansen L. G., Warwick W. J. A fluorometric micromethod for serum vitamin A. Am J Clin Pathol. 1968 Oct;50(4):525–529. doi: 10.1093/ajcp/50.4_ts.525. [DOI] [PubMed] [Google Scholar]
  13. Harries J. T., Muller D. P. Absorption of different doses of fat soluble and water miscible preparations of vitamin E in children with cystic fibrosis. Arch Dis Child. 1971 Jun;46(247):341–344. doi: 10.1136/adc.46.247.341. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. Hubbard V. S., Farrell P. M., di Sant'Agnese P. A. 25-Hydroxycholecalciferol levels in patients with cystic fibrosis. J Pediatr. 1979 Jan;94(1):84–86. doi: 10.1016/s0022-3476(79)80362-5. [DOI] [PubMed] [Google Scholar]
  15. Hume R., Weyers E. Changes in leucocyte ascorbic acid during the common cold. Scott Med J. 1973 Jan;18(1):3–7. doi: 10.1177/003693307301800102. [DOI] [PubMed] [Google Scholar]
  16. Kanai M., Raz A., Goodman D. S. Retinol-binding protein: the transport protein for vitamin A in human plasma. J Clin Invest. 1968 Sep;47(9):2025–2044. doi: 10.1172/JCI105889. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. Kopel F. B. Gastrointestinal manifestations of cystic fibrosis. Gastroenterology. 1972 Mar;62(3):483–491. [PubMed] [Google Scholar]
  18. LEVIN S., GORDON M. H., NITOWSKY H. M., GOLDMAN C., DI SANT'AGNESE P., GORDON H. H. Studies of tocopherol deficiency in infants and children. VI. Evaluation of muscle strength and effect of tocopherol administration in children with cystic fibrosis. Pediatrics. 1961 Apr;27:578–588. [PubMed] [Google Scholar]
  19. Lapey A., Kattwinkel J., Di Sant'Agnese P. A., Laster L. Steatorrhea and azotorrhea and their relation to growth and nutrition in adolescents and young adults with cystic fibrosis. J Pediatr. 1974 Mar;84(3):328–334. doi: 10.1016/s0022-3476(74)80712-2. [DOI] [PubMed] [Google Scholar]
  20. McCombs M. L. Research in cystic fibrosis: a review. Tex Rep Biol Med. 1973 Winter;31(4):615–629. [PubMed] [Google Scholar]
  21. Mitchell-Heggs P., Mearns M., Batten J. C. Cystic fibrosis in adolescents and adults. Q J Med. 1976 Jul;45(179):479–504. [PubMed] [Google Scholar]
  22. Moran J. R., Greene H. L. The B vitamins and vitamin C in human nutrition. I. General considerations and 'obligatory' B vitamins. Am J Dis Child. 1979 Feb;133(2):192–199. doi: 10.1001/archpedi.1979.02130020084018. [DOI] [PubMed] [Google Scholar]
  23. Nichoalds G. E. Assessment of status riboflavin nutriture by assay of erythrocyte glutathione reductase activity. Clin Chem. 1974 May;20(5):624–628. [PubMed] [Google Scholar]
  24. Orenstein D. M., Boat T. F., Stern R. C., Tucker A. S., Charnock E. L., Matthews L. W., Doershuk C. F. The effect of early diagnosis and treatment in cystic fibrosis: a seven-year study of 16 sibling pairs. Am J Dis Child. 1977 Sep;131(9):973–975. doi: 10.1001/archpedi.1977.02120220039005. [DOI] [PubMed] [Google Scholar]
  25. Peterson P. A., Rask L., Ostberg L., Andersson L., Kamwendo F., Pertoft H. Studies on the transport and cellular distribution of vitamin A in normal and vitamin A-deficient rats with special reference to the vitamin A-binding plasma protein. J Biol Chem. 1973 Jun 10;248(11):4009–4022. [PubMed] [Google Scholar]
  26. Poskitt E. M., Cole T. J., Lawson D. E. Diet, sunlight, and 25-hydroxy vitamin D in healthy children and adults. Br Med J. 1979 Jan 27;1(6158):221–223. doi: 10.1136/bmj.1.6158.221. [DOI] [PMC free article] [PubMed] [Google Scholar]
  27. SCHOUTEN H., STATIUSVANEPS L. W., STRUYKERBOUDIER A. M. TRANSKETOLASE IN BLOOD. Clin Chim Acta. 1964 Nov;10:474–476. doi: 10.1016/0009-8981(64)90179-2. [DOI] [PubMed] [Google Scholar]
  28. SHWACHMAN H., KULCZYCKI L. L. Long-term study of one hundred five patients with cystic fibrosis; studies made over a five- to fourteen-year period. AMA J Dis Child. 1958 Jul;96(1):6–15. doi: 10.1001/archpedi.1958.02060060008002. [DOI] [PubMed] [Google Scholar]
  29. Scott J., Elias E., Moult P. J., Barnes S., Wills M. R. Rickets in adult cystic fibrosis with myopathy, pancreatic insufficiency and proximal renal tubular dysfunction. Am J Med. 1977 Sep;63(3):488–492. doi: 10.1016/0002-9343(77)90290-x. [DOI] [PubMed] [Google Scholar]
  30. Shwachman H. Gastrointestinal manifestations of cystic fibrosis. Pediatr Clin North Am. 1975 Nov;22(4):787–805. doi: 10.1016/s0031-3955(16)33208-4. [DOI] [PubMed] [Google Scholar]
  31. Shwachman H., Redmond A., Khaw K. T. Studies in cystic fibrosis. Report of 130 patients diagnosed under 3 months of age over a 20-year period. Pediatrics. 1970 Sep;46(3):335–343. [PubMed] [Google Scholar]
  32. Skinner R. K., Wills M. R. Serum 25-hydroxyvitamin D assay. Evalution of chromatographic and non-chromatographic procedures. Clin Chim Acta. 1977 Nov 1;80(3):543–554. doi: 10.1016/0009-8981(77)90149-8. [DOI] [PubMed] [Google Scholar]
  33. Smith F. R., Goodman D. S. Vitamin A transport in human vitamin A toxicity. N Engl J Med. 1976 Apr 8;294(15):805–808. doi: 10.1056/NEJM197604082941503. [DOI] [PubMed] [Google Scholar]
  34. Smith F. R., Underwood B. A., Denning C. R., Varma A., Goodman D. S. Depressed plasma retinol-binding protein levels in cystic fibrosis. J Lab Clin Med. 1972 Sep;80(3):423–433. [PubMed] [Google Scholar]
  35. Stanulović M., Miletić D., Sttock A. Die Diagnostik des Vitamin B6-Mangels auf Grund der Bestimmung von erythrocytärer L-Aspartat:2-Oxoglutarat Aminotranspherase (Glutamat-Oxalacetat-Transaminase) und ihrer Stimulation in vitro mit Pyridoxal-5'-Phosphat. Clin Chim Acta. 1967 Sep;17(3):353–362. doi: 10.1016/0009-8981(67)90209-4. [DOI] [PubMed] [Google Scholar]
  36. Thomas W. R., Holt P. G. Vitamin C and immunity: an assessment of the evidence. Clin Exp Immunol. 1978 May;32(2):370–379. [PMC free article] [PubMed] [Google Scholar]
  37. Torstenson O. L., Humphrey G. B., Edson J. R., Warwick W. J. Cystic fibrosis presenting with severe hemorrhage due to vitamin K malabsorption: a report of three cases. Pediatrics. 1970 May;45(5):857–861. [PubMed] [Google Scholar]
  38. Walker B. E., Kelleher J., Davies T., Losowsky M. S. Chemical faecal fat using single stools. Scand J Gastroenterol. 1971;6(3):277–280. doi: 10.3109/00365527109180708. [DOI] [PubMed] [Google Scholar]
  39. Walters T. R., Koch H. F. Hemorrhagic diathesis and cystic fibrosis in infancy. Am J Dis Child. 1972 Nov;124(5):641–642. doi: 10.1001/archpedi.1972.02110170019002. [DOI] [PubMed] [Google Scholar]
  40. Warwick W. J., Pogue R. E. The prognosis for children with cystic fibrosis based on reasoned approaches to therapy: past, present, and future. J Asthma Res. 1968 Jun;5(4):277–284. doi: 10.3109/02770906809100345. [DOI] [PubMed] [Google Scholar]
  41. Wood R. E. Cystic fibrosis: diagnosis, treatment, and prognosis. South Med J. 1979 Feb;72(2):189–202. [PubMed] [Google Scholar]

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